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Erdheim-Chester disease

Introduction

Synonyms and keywords: Erdheim-Chester syndrome; polyostotic sclerosing histiocytosis; ECD

Overview

Overview

Erdheim-Chester disease is a rare form of non-Langerhans-cell histiocytosis. Usually, onset is in middle age. The disease involves an infiltration of lipid-laden macrophages, multinucleated giant cells, an inflammatory infiltrate of lymphocytes and histiocytes in the bone marrow, and a generalized sclerosis of the long bones.[1]

References

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Historical Perspective

Overview

Historical Perspective

The first case of ECD was reported by W. Chester and Jakob Erdheim in 1930.[1]

References

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Pathophysiology

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References

Causes

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References

Differentiating Erdheim-Chester disease from other Diseases

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References

Epidemiology and Demographics

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Overview

Epidemiology and Demographics

Age

ECD affects predominantly adults, with a mean age of 53 years.[1]

References

  1. ↑ Veyssier-Belot C, Cacoub P, Caparros-Lefebvre D; et al. (1996). “Erdheim-Chester disease. Clinical and radiologic characteristics of 59 cases”. Medicine (Baltimore). 75 (3): 157–69. PMID 8965684.

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Natural History, Complications and Prognosis

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References

Diagnosis

Diagnosis

History and Symptoms | Physical Examination | Laboratory Findings | Electrocardiogram | X Ray | CT | MRI | Echocardiography or Ultrasound | Other Imaging Findings | Other Diagnostic Studies

Treatment

Treatment

Medical Therapy | Surgery | Cost-Effectiveness of Therapy | Future or Investigational Therapies

Case Studies

Case Studies

Case #1

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]